Abstract：
:The goal of this study is to define the clinical features, pathogenesis and key investigation findings of fasciculation anxiety syndrome in clinicians (FASICS). Twenty consecutive clinicians presenting with fasciculations were prospectively assessed with serial clinical and neurophysiological evaluations. Clinicians with fasciculations formed three groups: 70 % of clinicians experienced symptomatic fasciculations and anxiety about the possibility of amyotrophic lateral sclerosis (ALS), termed FASICS; a further 15 % of clinicians experienced fasciculations associated with cramps and consistent with cramp-fasciculation syndrome (CFS). The final 15 % of clinicians presented with fasciculations associated with sensory symptoms or muscle weakness and were diagnosed with neuropathy (10 %) and ALS (5 %). In FASICS, fasciculations most often involved the lower limbs, without evidence of muscle weakness on clinical examination. Exercise, stress, fatigue and caffeine consumption were identified as common exacerbating factors. Neurophysiological studies confirmed normal nerve conduction studies and the presence of simple fasciculations, without acute denervation or neurogenic motor unit changes. Antibodies to voltage-gated potassium channels were assayed in each clinician and were not detected, and systemic autoantibodies were detected only in clinicians with features of CFS. FASICS is a disorder common among physicians presenting for evaluation of fasciculations. The present study delineates the diagnostic features of FASICS and contrasts the clinical presentation with other causes of fasciculations in clinicians.
journal_name
J Neuroljournal_title
Journal of neurologyauthors
Simon NG,Kiernan MCdoi
10.1007/s00415-013-6856-8subject
Has Abstractpub_date
2013-07-01 00:00:00pages
1743-7issue
7eissn
0340-5354issn
1432-1459journal_volume
260pub_type
杂志文章abstract：:Elevated uric acid levels have recently been found to be associated with slower disease progression in Parkinson's disease, Huntington's disease, multiple system atrophy, and mild cognitive impairment. The aim of this study is to determine whether serum uric acid levels predict survival in amyotrophic lateral sclerosi...
journal_title：Journal of neurology
pub_type： 杂志文章,多中心研究
doi：10.1007/s00415-012-6440-7
更新日期：2012-09-01 00:00:00
abstract：:Corticobasal degeneration (CBD) was first described by Rebeiz et al. in 1967, and was called corticodentatonigral degeneration with neuronal achromasia [1]. Since then, our knowledge of the clinical features and underlying tau pathology has grown tremendously. Clinical antemortem diagnosis of CBD pathology remains cha...
journal_title：Journal of neurology
pub_type： 历史文章,杂志文章
doi：10.1007/s00415-017-8644-3
更新日期：2018-02-01 00:00:00
abstract：BACKGROUND:Dermic fibroblasts have been proposed as a potential genetic-ALS cellular model. This study aimed to explore whether dermic fibroblasts from patients with sporadic-ALS (sALS) recapitulate alterations typical of ALS motor neurons and exhibit abnormal DNA-damage response. METHODS:Dermic fibroblasts were obtai...
journal_title：Journal of neurology
pub_type： 杂志文章
doi：10.1007/s00415-020-09704-8
更新日期：2020-05-01 00:00:00
abstract：CONTEXT:Polyneuropathy is a common disease and is more prevalent (at least 3 %) in elderly people. However, routine neurological examination of healthy elderly people may show distal sensory loss and absent tendon reflexes, which can obscure the distinction from polyneuropathy. OBJECTIVE:To investigate the relation be...
journal_title：Journal of neurology
pub_type： 杂志文章,meta分析
doi：10.1007/s00415-005-0064-0
更新日期：2006-05-01 00:00:00
abstract：:The purpose of this study was to evaluate the contribution of posterior circulation to memory function by comparing memory scores between patients with and without a foetal-type posterior cerebral artery (FTP) during the intracarotid amobarbital procedure (IAP) in epilepsy patients. Patients undergoing bilateral IAP b...
journal_title：Journal of neurology
pub_type： 杂志文章
doi：10.1007/s00415-011-6391-4
更新日期：2012-08-01 00:00:00
abstract：:Isolated floccular infarction is extremely rare, and impairments of the vestibulo-ocular reflex (VOR) have not been explored in humans with isolated floccular lesions. The purpose of this study was to examine and report selective impairment of VOR in response to high acceleration using head impulse in a patient with i...
journal_title：Journal of neurology
pub_type： 杂志文章
doi：10.1007/s00415-013-6837-y
更新日期：2013-06-01 00:00:00
abstract：:Epileptic syndromes are distinctive disorders with specific features, which when taken together, permit a specific diagnosis. There is actually a debate on that medial temporal lobe epilepsy with hippocampal sclerosis is an epileptic syndrome. To address this issue, we searched for discriminative semiological features...
journal_title：Journal of neurology
pub_type： 杂志文章
doi：10.1007/s00415-017-8441-z
更新日期：2017-05-01 00:00:00
abstract：PURPOSE:Epileptogenic foci exhibit disturbed function at the level of the benzodiazepine receptor. The aim of our study was to investigate the incidence of focal reductions of temporal benzodiazepine receptor binding (BRB) as assessed by scintigraphy with 123I-iomazenil in patients with denovo temporal lobe epilepsy (T...
journal_title：Journal of neurology
pub_type： 杂志文章
doi：10.1007/s004150170136
更新日期：2001-07-01 00:00:00
abstract：:To clarify the changes of cervical (cVEMP) and ocular (oVEMP) vestibular evoked myogenic potentials induced by air-conducted sound (ACS) and bone-conducted vibration (BCV) in patients with vestibular neuritis (VN), patients with VN (n = 30) and normal controls (n = 45) underwent recording of cVEMP and oVEMP in respons...
journal_title：Journal of neurology
pub_type： 杂志文章
doi：10.1007/s00415-013-6953-8
更新日期：2013-08-01 00:00:00
abstract：:Twenty-nine cases of both clinically and neuropathologically diagnosed dementia with Lewy bodies (DLB) were retrospectively examined for autonomic symptoms. Twenty-eight cases showed some kind of autonomic dysfunction. Urinary incontinence (97 %) and constipation (83 %) were the two most common. Although urinary reten...
journal_title：Journal of neurology
pub_type： 杂志文章
doi：10.1007/s00415-003-1029-9
更新日期：2003-05-01 00:00:00
abstract：BACKGROUNDS:Wake-Up Stroke (WUS) patients are generally excluded from thrombolytic therapy (rTPA) due to the unknown time of stroke onset. This study aimed to investigate the effects of rTPA in WUS patients during every day clinical scenarios, by measuring ischemic lesion volume and functional outcomes compared to non-...
journal_title：Journal of neurology
pub_type： 杂志文章
doi：10.1007/s00415-019-09603-7
更新日期：2020-03-01 00:00:00
abstract：:Evoked urethral and anal responses are produced by electrical stimulation of penis, bladder neck and anus. Latency and duration of the responses after bladder neck and anal stimulation are greatly dependent on stimulus parameters, suggesting a polysynaptic reflex; penile stimulation probably involves an oligosynaptic ...
journal_title：Journal of neurology
pub_type： 杂志文章
doi：10.1007/BF00313567
更新日期：1982-01-01 00:00:00
abstract：:Autonomic dysfunction is frequently observed in patients with multiple sclerosis (MS), but clinical studies disagree on the frequency and type of abnormalities in autonomic function tests. Orthostatic dizziness (OD) has been reported in up to 49% of patients, but the pathophysiological mechanisms are poorly understood...
journal_title：Journal of neurology
pub_type： 杂志文章
doi：10.1007/s004150050407
更新日期：1999-07-01 00:00:00
abstract：:Gait variability has potential utility as a predictive measure of dysfunction in Parkinson's disease (PD). Current understanding implicates non-dopaminergic pathways. This study investigated the explanatory characteristics of gait variability in PD on and off medication under single and dual task conditions. Fifty peo...
journal_title：Journal of neurology
pub_type： 杂志文章
doi：10.1007/s00415-010-5789-8
更新日期：2011-04-01 00:00:00
abstract：OBJECTIVES:Resistance to activated protein C (APC) is the most frequent cause of thrombophilia and a well known risk factor for deep and cerebral vein thrombosis. Its causative role in ischaemic stroke is still a matter of debate. We undertook this study to determine the prevalence of APC-resistance in a cohort of cons...
journal_title：Journal of neurology
pub_type： 临床试验,杂志文章
doi：10.1007/s004150170117
更新日期：2001-08-01 00:00:00
abstract：:We describe three patients with unilateral asterixis. One diabetic patient with a moderately-sized haematoma in the left putamen initially developed bilateral and symmetrical asterixis, which became confined to the right side as his diabetes mellitus was controlled. Two patients showed unilateral asterixis due to haem...
journal_title：Journal of neurology
pub_type： 杂志文章
doi：10.1007/BF00314766
更新日期：1990-12-01 00:00:00
abstract：:Surface antigens on peripheral blood lymphocytes from myasthenia gravis patients were investigated. The expression of DR+ and CD8+/DR+ T lymphocytes was increased and the expression of CD4+ T cells reduced. Neither thymectomy, clinical condition nor anti-acetylcholine receptor antibody titre correlated with any of the...
journal_title：Journal of neurology
pub_type： 杂志文章
doi：10.1007/BF00863771
更新日期：1994-02-01 00:00:00
abstract：BACKGROUND:Therapy of autoimmune diseases of the central and peripheral nervous system with intravenous IgG immunoglobulin (IVIg) is well established. Since IVIg is produced from pooled human plasma, autoantibodies can be found in IVIg products and, accordingly, in patient sera after transfusion. The de novo evidence o...
journal_title：Journal of neurology
pub_type： 杂志文章
doi：10.1007/s00415-019-09614-4
更新日期：2020-03-01 00:00:00
abstract：:A linkage and association of the CD45 (protein-tyrosine phosphatase, receptor-type C) C77G polymorphism and multiple sclerosis (MS) has been found in some studies but not in others. We analysed the C77G polymorphism in MS patients from the genetically homogeneous population of Sardinia. Using the transmission disequil...
journal_title：Journal of neurology
pub_type： 杂志文章
doi：10.1007/s00415-004-0485-1
更新日期：2004-09-01 00:00:00
abstract：:The Analog Model was developed for use in patients with multiple sclerosis (MS) at an international roundtable meeting in 2001. Three key disease components are monitored, namely relapses, disability progression and magnetic resonance imaging activity. Its use provides a rapid, straightforward means of assessing the e...
journal_title：Journal of neurology
pub_type： 杂志文章,评审
doi：10.1007/s00415-004-1512-y
更新日期：2004-09-01 00:00:00
abstract：:The symptomatology of 18 patients with the severest clinical course after subarachnoid hemorrhage (SAH) is described and analyzed. Seventeen patients died, five with an acute bulbar syndrome with cardiac arrest, and 12 with irreversible breakdown of brain function. One patient had an apallic syndrome with minimal sign...
journal_title：Journal of neurology
pub_type： 杂志文章
doi：10.1007/BF00313005
更新日期：1979-01-01 00:00:00
abstract：:The concentration of neopterin was measured in serum samples taken from individuals infected with HTLV-I: 5 from asymptomatic individuals, 1 from a patient with adult T-cell leukaemia and 30 from patients with tropical spastic paraparesis (TSP). In addition, cerebrospinal fluid (CSF) was available from 22 of the TSP p...
journal_title：Journal of neurology
pub_type： 杂志文章
doi：10.1007/BF00810351
更新日期：1992-05-01 00:00:00
abstract：:Multiple system atrophy and progressive supranuclear palsy are disabling neurodegenerative disorders, also known as atypical parkinsonian syndromes. Currently, no health economic evaluations of these diseases are available. The objective of this study was to evaluate disease-related costs in German patients with multi...
journal_title：Journal of neurology
pub_type： 杂志文章
doi：10.1007/s00415-011-6028-7
更新日期：2011-10-01 00:00:00
abstract：:Four patients with aphasia due to small circumscribed thalamic lesions are presented. A review of the literature on thalamic aphasia revealed 16 similar cases. While the general consensus that only left-sided thalmic lesions are associated with aphasia is confirmed, analysis of the sites of the thalamic infarctions an...
journal_title：Journal of neurology
pub_type： 杂志文章,评审
doi：10.1007/BF00314212
更新日期：1989-01-01 00:00:00
abstract：:Myasthenia gravis (MG) is the archetypic disorder of both the neuromuscular junction and autoantibody-mediated disease. In most patients, IgG1-dominant antibodies to acetylcholine receptors cause fatigable weakness of skeletal muscles. In the rest, a variable proportion possesses antibodies to muscle-specific tyrosine...
journal_title：Journal of neurology
pub_type： 杂志文章,评审
doi：10.1007/s00415-015-7963-5
更新日期：2016-04-01 00:00:00
abstract：:Normal pressure hydrocephalus (NPH) is characterised by gait disturbance, urinary incontinence and dementia. Even though dementia is a cardinal symptom of NPH, there is few data available concerning cognitive functioning. The aim of this observational case-control study was to evaluate the use of neuropsychological (N...
journal_title：Journal of neurology
pub_type： 杂志文章
doi：10.1007/s00415-014-7489-2
更新日期：2014-12-01 00:00:00
abstract：:Limb-girdle muscular dystrophies (LGMDs) are genetically heterogeneous and the diagnostic work-up including conventional genetic testing using Sanger sequencing remains complex and often unsatisfactory. We performed targeted sequencing of 23 LGMD-related genes and 15 genes in which alterations result in a similar phen...
journal_title：Journal of neurology
pub_type： 杂志文章
doi：10.1007/s00415-016-8036-0
更新日期：2016-04-01 00:00:00
abstract：:The objective of this study was to assess the effect of secukinumab, a monoclonal antibody that inhibits interleukin (IL)-17A, on number of new active brain magnetic resonance imaging (MRI) lesions in subjects with relapsing-remitting multiple sclerosis (MS). Subjects (N = 73) were randomized 1:1 to secukinumab 10 mg/...
journal_title：Journal of neurology
pub_type： 杂志文章,多中心研究,随机对照试验
doi：10.1007/s00415-016-8128-x
更新日期：2016-07-01 00:00:00
abstract：:Cerebellar syndrome is one of the most disabling developments in multiple sclerosis (MS). In neurodegenerative disorders, cerebellar syndrome is thought to be related to a neurochemical deficit of 5-hydroxytryptamine (5-HT). Previous studies found that a levorotatory form of 5-hydroxytryptophan, a 5-HT precursor, and ...
journal_title：Journal of neurology
pub_type： 临床试验,杂志文章,随机对照试验
doi：10.1007/s00415-003-0176-3
更新日期：2003-10-01 00:00:00
abstract：OBJECTIVES:Evidences from either small series or spontaneous reporting are accumulating that SARS-CoV-2 involves the Nervous Systems. The aim of this study is to provide an extensive overview on the major neurological complications in a large cohort of COVID-19 patients. METHODS:Retrospective, observational analysis o...
journal_title：Journal of neurology
pub_type： 杂志文章
doi：10.1007/s00415-020-10251-5
更新日期：2020-10-07 00:00:00
